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2型早期黄斑毛细血管扩张症患者眼部结构与血管异常的多层视网膜对应关系

Multilayer Retinal Correspondence of the Structural and Vascular Anomalies in Eyes With Early Macular Telangiectasia Type 2.

作者信息

Krivosic Valérie, Dobbels Zoe, Duliere Cedric, Zureik Abir, Tadayoni Ramin, Gaudric Alain

机构信息

Ophthalmology Department, AP-HP, Hôpital Lariboisière, Université Paris Cité, Paris, France and Centre de Référence des Maladies Vasculaires Rares du Cerveau et de l'Œil (CERVCO), Hôpital Lariboisière, APHP, Paris, France.

出版信息

Invest Ophthalmol Vis Sci. 2024 Sep 3;65(11):24. doi: 10.1167/iovs.65.11.24.

DOI:10.1167/iovs.65.11.24
PMID:39283616
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11407475/
Abstract

PURPOSE

To assess the correspondence between interdigitation zone (IZ) reflectivity, ellipsoid zone (EZ) loss, inner retinal layer reflectivity, patterns of capillary dilation, and telangiectasia in eyes with early macular telangiectasia type 2 (MacTel).

PATIENTS AND METHODS

Twenty-eight eyes of 22 patients with grade 0-2 MacTel (according to the MacTel project classification) and 28 healthy control eyes were included in this study. Multimodal imaging, including optical coherence tomography (OCT) angiography, adaptive optics flood illumination ophthalmoscopy (AO-FIO) and blue light reflectance (BLR), was performed. The EZ, IZ, and outer plexiform layer (OPL) were analyzed on the structural OCT C-scans. The vascular density (VD) was measured on the binarized and skeletonized angiograms of the superficial vascular plexus and deep capillary complex. The vascular diameter index (VDI) was calculated by dividing the binarized VD by the skeletonized VD.

RESULTS

On AO-FIO, cone density in the MacTel zone was significantly lower in MacTel eyes than in controls, even in areas located outside the EZ loss (P < 0.001). A distinctive pattern of IZ reflectivity attenuation extended beyond the area of EZ attenuation. The shape and size of a strong OPL hyper-reflectivity corresponded to the MacTel white area (MacTel zone) seen on BLR. Capillary dilation and rarefaction were colocalized with this area, extending beyond visible telangiectasia. The VDI was higher in MacTel eyes than in controls (P < 0.001).

CONCLUSIONS

These findings suggest that in early MacTel eyes, photoreceptor signal alteration, OPL hyper-reflectivity, and capillary dilation, potentially associated with Müller cell dysfunction, precede the EZ loss.

摘要

目的

评估2型早期黄斑毛细血管扩张症(MacTel)患者眼睛的指状交叉区(IZ)反射率、椭圆体区(EZ)缺失、视网膜内层反射率、毛细血管扩张模式和毛细血管扩张之间的相关性。

患者和方法

本研究纳入了22例0-2级MacTel患者(根据MacTel项目分类)的28只眼睛和28只健康对照眼睛。进行了多模态成像,包括光学相干断层扫描(OCT)血管造影、自适应光学泛光照明检眼镜检查(AO-FIO)和蓝光反射率(BLR)检查。在结构性OCT C扫描上分析EZ、IZ和外丛状层(OPL)。在浅表血管丛和深部毛细血管复合体的二值化和骨架化血管造影上测量血管密度(VD)。通过将二值化VD除以骨架化VD来计算血管直径指数(VDI)。

结果

在AO-FIO检查中,MacTel患者眼睛的MacTel区视锥细胞密度显著低于对照组,即使在EZ缺失区域以外的区域也是如此(P<0.001)。IZ反射率衰减的独特模式延伸到EZ衰减区域之外。强OPL高反射率的形状和大小与BLR上所见的MacTel白色区域(MacTel区)相对应。毛细血管扩张和稀疏与该区域共定位,延伸到可见的毛细血管扩张之外。MacTel患者眼睛的VDI高于对照组(P<0.001)。

结论

这些发现表明,在早期MacTel患者眼睛中,与Müller细胞功能障碍潜在相关的光感受器信号改变、OPL高反射率和毛细血管扩张先于EZ缺失出现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/01f9/11407475/5875e16bbc29/iovs-65-11-24-f007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/01f9/11407475/1b7447a74d49/iovs-65-11-24-f001.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/01f9/11407475/5875e16bbc29/iovs-65-11-24-f007.jpg

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